Origen aórtico anómalo de las arterias coronarias / Abnormal aortic origin of coronary arteries

Resumen Introducción: El origen aórtico anómalo de las arterias coronarias tiene una prevalencia estimada del 0.02-5.7% y están potencialmente involucradas con complicaciones en su evolución natural o aquellos que requieran procedimientos intervencionistas hemodinámico y/o quirúrgicos. Puede asociarse a muerte súbita o dañarse durante intervenciones sobre el anillo mitral, aórtico, pulmonar o el cierre percutáneo de un defecto septal interauricular. Objetivo: identificar estos pacientes por diferentes técnicas de imágenes como el ecocardiograma Doppler color transtorácico (ETT), angiotomografía o angiografía coronaria. Métodos: Las técnicas de imágenes utilizadas para la detección de anomalías coronarias fueron el ETT, angiotomografía coronaria multicorte o angiografía coronaria convencional de acuerdo con lo universalmente aceptado. Resultados: Estudio prospectivo realizado desde enero del 2020 a junio del 2021. Se identificaron 15 pacientes y en 12/15 la sospecha fue por ETT y en los tres restantes por angiotomografía coronaria. La arteria circunfleja fue la más involucrada en forma aislada o asociada a otra anomalía coronaria (12/15 pacientes) y en los tres casos restantes la coronaria anómala tuvo un trayecto interarterial, siendo las arterias coronarias derecha y la descendente anterior las afectadas. Conclusiones: El subdiagnóstico por ETT de las anomalías de las arterias coronarias puede deberse a la dificultad para su visualización, sobre todo en la población adulta. Su detección es crucial, ya que puede generar muerte súbita asociada a isquemia miocárdica y arritmias graves o complicar procedimientos intervencionistas sobre el septum interauricular o sobre los anillos mitral, pulmonar y/o aórtico.

Abstract Introduction: The anomalous aortic origin of the coronary arteries has an estimated prevalence of 0.02-5.7%. It can be associated with sudden death when it has an interarterial or intramural pathway or be damaged during interventions on the mitral, pulmonary and/or aortic annulus or percutaneous closure of an interatrial septal defect. Objective: To identify these patients by imaging techniques such as transthoracic color Doppler echocardiography (TTE), computed tomography (CT) multislice angiography or coronary angiography. Methods: The imaging techniques used for the detection of coronary anomalies were TTE, multislice coronary angiography or coronary angiography according to what is generally accepted. Results: Fifteen patients were identified; in 12 of them the suspicion was due to TTE and in the remaining 3, CT multislice angiography was diagnostic. The circumflex artery was the coronary artery most involved, associated or not with another coronary anomaly (12/15 patients) and in the other three cases, the anomalous coronary artery had an interarterial course, with the right coronary arteries and the anterior descending coronary arteries being involved. Conclusions: The under diagnosis by TTE of coronary artery abnormalities may be due to the difficulty of visualization that is accentuated with age. Their detection is crucial because they can both, lead to sudden death associated with an intramural and/or interarterial pathway and complicate an interventional procedure on the interatrial septum or within the mitral, pulmonary and/or aortic rings.

[Abnormal aortic origin of coronary arteries]. / Origen aórtico anómalo de las arterias coronarias.

Introduction: The anomalous aortic origin of the coronary arteries has an estimated prevalence of 0.02-5.7%. It can be associated with sudden death when it has an interarterial or intramural pathway or be damaged during interventions on the mitral, pulmonary and/or aortic annulus or percutaneous closure of an interatrial septal defect. Objective: To identify these patients by imaging techniques such as transthoracic color Doppler echocardiography (TTE), computed tomography (CT) multislice angiography or coronary angiography. Methods: The imaging techniques used for the detection of coronary anomalies were TTE, multislice coronary angiography or coronary angiography according to what is generally accepted. Results: Fifteen patients were identified; in 12 of them the suspicion was due to TTE and in the remaining 3, CT multislice angiography was diagnostic. The circumflex artery was the coronary artery most involved, associated or not with another coronary anomaly (12/15 patients) and in the other three cases, the anomalous coronary artery had an interarterial course, with the right coronary arteries and the anterior descending coronary arteries being involved. Conclusions: The under diagnosis by TTE of coronary artery abnormalities may be due to the difficulty of visualization that is accentuated with age. Their detection is crucial because they can both, lead to sudden death associated with an intramural and/or interarterial pathway and complicate an interventional procedure on the interatrial septum or within the mitral, pulmonary and/or aortic rings.

Introducción: El origen aórtico anómalo de las arterias coronarias tiene una prevalencia estimada del 0.02-5.7% y están potencialmente involucradas con complicaciones en su evolución natural o aquellos que requieran procedimientos intervencionistas hemodinámico y/o quirúrgicos. Puede asociarse a muerte súbita o dañarse durante intervenciones sobre el anillo mitral, aórtico, pulmonar o el cierre percutáneo de un defecto septal interauricular. Objetivo: Identificar estos pacientes por diferentes técnicas de imágenes como el ecocardiograma Doppler color transtorácico (ETT), angiotomografía o angiografía coronaria. Métodos: Las técnicas de imágenes utilizadas para la detección de anomalías coronarias fueron el ETT, angiotomografía coronaria multicorte o angiografía coronaria convencional de acuerdo con lo universalmente aceptado. Resultados: Estudio prospectivo realizado desde enero del 2020 a junio del 2021. Se identificaron 15 pacientes y en 12/15 la sospecha fue por ETT y en los tres restantes por angiotomografía coronaria. La arteria circunfleja fue la más involucrada en forma aislada o asociada a otra anomalía coronaria (12/15 pacientes) y en los tres casos restantes la coronaria anómala tuvo un trayecto interarterial, siendo las arterias coronarias derecha y la descendente anterior las afectadas. Conclusiones: El subdiagnóstico por ETT de las anomalías de las arterias coronarias puede deberse a la dificultad para su visualización, sobre todo en la población adulta. Su detección es crucial, ya que puede generar muerte súbita asociada a isquemia miocárdica y arritmias graves o complicar procedimientos intervencionistas sobre el septum interauricular o sobre los anillos mitral, pulmonar y/o aórtico.

Sufficient versus deficient rims during percutaneous closure of ostium secundum type atrial septal defect: A systematic review and meta-analysis.

BACKGROUND: The aim of this meta-analysis was to compare the efficacy and adverse events of percutaneous occlusion among patients with sufficient and deficient rims. METHODS: A systematic review of all articles published in the Pubmed, MEDLINE and Google Scholar databases was performed. Odds ratio (OR) and 95% CI were used as a measure of effect of the combination of studies. I2 with 95% CI was estimated to assess study heterogeneity. For the meta-analysis, a random effects model was used. RESULTS: The systematic search identified ten studies which included 4355 patients; 2661 of those had sufficient rim and the remaining 1694 patients showed some rim deficiency. Implant failure rate was 4.13% CI 95% 3.53-4.72%. Compared to frequency of failures in the group with a deficient rim (5.43% CI 95% 4.35-6.50%), implant failure in patients with a sufficient rim was significantly lower (3.30% CI 95% 2.62-3.97%), OR 2.27 CI 1.34-3.83 (p 0.002). The combined adverse events were 5.19% CI 95% 4.22-6.35% vs 2.7% CI 95% 2.08-3.31% in the deficient vs sufficient rim groups respectively (OR 2.21 CI 0.93-5.29; p 0.07). Implant failures and adverse events were more frequent in patients with posterior inferior rim deficiency. CONCLUSION: Patients presenting a posteroinferior rim deficiency are associated to both, an increased incidence of closure failure and a combined adverse events occurrence. More studies on posterior rim deficiency are necessary to ensure the feasibility and safety of the percutaneous approach.

Percutaneous Closure of Ruptured Sinus of Valsalva Aneurysm in a Child Using a Patent Ductus Arteriosus Device.

Ruptured sinus of Valsalva aneurysm is rare, although if left untreated, potentially fatal disease. Surgical approach has been the main treatment in most series; nevertheless, percutaneous closure has been described in selected cases. We report a 5-year-old boy presenting with rapid clinical deterioration who underwent percutaneous closure using a patent ductus arteriosus device, with the resolution of symptoms. Descriptions of this technique being utilized in children are infrequent in the literature.

Tetralogy of Fallot Repair After Neonatal Right Ventricular Outflow Tract Stenting: Initial Multicenter Experience in Argentina.

Initial management of patients with tetralogy of Fallot, unfavorable anatomy, and reduced pulmonary blood flow is controversial and continues to be a clinical challenge. Pulmonary to systemic shunt anastomosis or primary correction in neonates and small infants is associated with higher morbimortality and increased number of reoperations. Initial right ventricle outflow tract stenting palliation has emerged as an attractive alternative. We report our experience in 14 patients operated on with tetralogy of Fallot and previous right ventricle outflow tract stenting from March 2018 to June 2022. All stented patients had pulmonary annulus and main pulmonary artery Z score ≤ -2.5. Surgical outcomes, complications, and mortality at 30 days were evaluated. Patient's age and weight at surgery were 5.9 months (2-17) and 6.1 kg (3.9-8.9), respectively. Stents were completely removed in 57.1% of patients. A transannular patch was placed in 10 patients, 3 patients required a right ventricle to pulmonary artery conduit due to coronary anomalies and in 1 patient, the pulmonary valve was preserved. Length of stay and ventilation time were 13.6 days (5-27) and 44.8 h (6-44), respectively. Mean time for right ventricle outflow tract stent implantation to surgical correction was 4 months (2-16). There was no mortality, and mean follow-up time of this cohort was 23.1 month (1-41). Surgical correction of severe tetralogy of Fallot after right ventricle outflow tract stenting is an effective alternative achievable without an increase in morbidity and mortality. Difficulty in stent extraction is related to the time since implantation. More number of patients and longer follow-up time are needed to confirm these initial results.

Comparación de resultados inmediatos y a mediano plazo de la anastomosis de Blalock-Taussig versus la colocación de stent en tracto de salida de ventrículo derecho en neonatos sintomáticos con tetralogía de Fallot / Comparison of immediate and mid-term outcomes of Blalock-Taussig shunt versus right ventricular outflow tract stenting in symptomatic neonates with tetralogy of Fallot

RESUMEN Introducción: El manejo inicial de neonatos con tetralogía de Fallot (TF), con síntomas asociados a una anatomía desfavorable y un flujo vascular pulmonar significativamente reducido es controversial, y un desafío clínico. Objetivo: Describir la evolución clínica y comparar el crecimiento de ramas pulmonares en neonatos con TF sintomáticos ingresados al Departamento de Cardiología del Hospital de Niños de Córdoba, desde marzo de 2011 hasta marzo de 2021, que recibieron anastomosis de Blalock-Taussig modificada (aBTm) versus colocación de stent en tracto de salida de ventrículo derecho (sTSVD). Material y métodos: Estudio retrospectivo, observacional. Se identificaron 113 pacientes con TF; 20 de ellos (18%) fueron neonatos sintomáticos y requirieron paliación inicial. Las variables categóricas se expresan como porcentaje; las continuas como mediana y rango intercuartilo (RIC). Un valor de p <0,05 se consideró significativo. Resultados: De los 20 pacientes incluidos en el estudio, 11 (55%) constituyen el grupo aBTm y 9 (45%) el grupo sTSVD. En el grupo aBTm la rama pulmonar derecha (RPD) pre paliación tenía un score Z -3 (RIC 4,20), que aumentó a -1,6 (RIC 1,56) (p = 0,11) post intervención; y la rama pulmonar izquierda (RPI) un score Z -2,5 (RIC 4,8) que se incrementó a -1,80 (RIC 2,36) (p = 0,44). En el grupo sTSVD la RPD pre paliación tuvo un score Z -3,45 (RIC 3,83) que aumentó a - 2,5 (RIC 3,58) (p = 0,021) y la RPI un score Z -4,10 (RIC 2,51) que se incrementó a -2,00 (RIC 3,75) (p = 0,011). La saturación de O2 (SO2) pre intervención fue 75% (RIC 6), y aumentó a 87% (RIC 9) en el grupo aBTm (p = 0,005); e inicialmente fue 75% (RIC 16) y aumentó a 91% (RIC 13) en el grupo sTSVD (p = 0,008). La mediana de estadía hospitalaria post procedimiento fue 10 días (RIC 11) en el grupo aBTm, y 6 (RIC 2) en el grupo sTSVD (p= 0,095). Conclusiones: En neonatos con TF sintomáticos, ambas estrategias paliativas mejoran la condición clínica. En los que recibieron sTSVD, se objetivó un crecimiento mayor de las ramas pulmonares. Mayor número de casos y seguimiento más largo serán necesarios para confirmar estos hallazgos.

ABSTRACT Background: The initial management of neonate patients with tetralogy of Fallot (TOF) associated with an unfavorable anatomy and significantly reduced pulmonary vascular flow is controversial and continues to be a clinical challenge. Objective: The aim of this study was to describe the clinical evolution and to compare pulmonary artery branch development in symptomatic neonatal TOF patients who received a modified Blalock Taussig shunt (mBT) versus right ventricular outflow tract stent placement (RVOTs) at the Department of Cardiology, Hospital de Niños de Córdoba, between March 2011 and March 2021. Methods: A retrospective, observational study identified 113 patients with TOF, 20 of which (18%) were symptomatic neonates requqiring initial palliative intervention. Categorical variables are expressed as percentage and continuous variables as median and interquartile range (IQR). A p value <0.05 was considered significant. Results: Among the 20 patients included in the study, 11 (55%) formed the mBT group and 9 (45%) the RVOTs group. In the mBT group, pre-palliative procedure right pulmonary artery (RPA) Z score was -3 (IQR 4.20) and increased to -1.6 (IQR 1.56) (p = 0.11) and left pulmonary artery (LPA) Z score of -2.5 (IQR 4.8) increased to -1.80 (IQR 2.36) (p = 0.44). In the RVOTs group, RPA Z score prior to palliative intervention was -3.45 (IQR 3.83) and increased to -2.5 (IQR 3.58) (p = 0.021) and LPA Z score of -4.10 (IQR 2.51) to -2.00 (IQR 3.75) (p = 0.011). Pre-intervention peripheral O2 saturation of 75% (IQR 6) increased to 87% (IQR 9) in the mBT group (p= 0.005) and from initially 75% (IQR 16) to 91% (IQR 13) in the RVOTs group (p= 0.008). Mean hospital stay after the procedure was 10 days (IQR 11) in the mBT group and 6 days (IQR 2) in the RVOTs group (p= 0.095). Conclusions: In symptomatic neonates with TOF, both palliative strategies improved the clinical condition. In patients who received RVOTs, there was greater development of pulmonary artery branches. A larger number of cases and longer-term follow-up will be necessary to confirm these findings.

Giant Right Ventricular Fibroma: Prenatal Diagnosis and Partial Resection in Early Infancy.

Congenital cardiac fibromas are very rare and prenatal diagnosis has been reported in just a few cases. We describe a four-month-old infant presenting a symptomatic giant right ventricular fibroma discovered during prenatal scanning at 33 weeks of gestation, which was confirmed after delivery on echocardiogram and cardiac magnetic resonance imaging. Due to progressive hemodynamic deterioration, partial surgical resection was performed and the patient recovered uneventfully. We report the successful management during early infancy of a giant cardiac fibroma prenatally diagnosed.

Usefulness of VExUS in a pediatric patient with Ebstein's anomaly / Utilidad de VExUS en paciente pediátrico portador de anomalía de Ebstein

The venous excess ultrasound grading system (VExUS) is a relatively new application of point of care ultrasound. It has been successfully used to monitor for fluid overload in adult post-operative cardiac surgery patients. It has not been described in the management of congenital cardiac disease. We present a novel use of VExUS to diagnose decompensated Ebstein's anomaly in a pediatric patient. A 13 year-old female with known stable Ebstein's anomaly presented with new onset fluid overload and oliguria. Using abdominal venous Doppler ultrasound and VExUS, we were able to quickly differentiate the cause of the patient's fluid overload as Ebstein's anomaly instead of a primary renal etiology. This is the first reported use of VExUS to diagnose acute fluid overload in a pediatric patient with Ebstein's anomaly. VExUS should be considered as a supplemental tool to diagnose fluid overload in other congenital cardiac diseases.

El sistema de gradiente por ultrasonidos de exceso venoso (VExUS), es una aplicación relativamente nueva de los ultrasonidos en el punto de cuidado. Esto ha sido utilizado exitosamente para monitorizar la sobrecarga de fluidos en pacientes adultos en el período posoperatorio de cirugía cardíaca. No ha sido descripto en el manejo de las cardiopatías congénitas. Nosotros presentamos un novedoso uso de VExUS para diagnosticar una anomalía de Ebstein descompensada en un paciente pediátrico. Paciente femenina de 13 años de edad con una anomalía de Ebstein compensada en su evolución, se presenta con sobrecarga de fluidos y oliguria. Usando ultrasonidos Doppler de vasos abdominales (VExUS), fue posible diferenciar rápidamente la causa de la sobrecarga de fluidos en una anomalía de Ebstein que provoca insuficiencia renal. Este el primer caso reportado del uso del VExUS para el diagnóstico de sobrecarga de fluidos en pacientes pediátricos con anomalía de Ebstein. El score VExUS debería ser considerado como método complementario en el diagnóstico de la sobrecarga de fluidos en otras cardiopatías congénitas.

Bailout procedure in obstructed supracardiac total anomalous pulmonary vein drainage / Procedimiento de rescate en drenaje venoso pulmonar anómalo total supracardíaco obstructivo

Abstract Total anomalous pulmonary venous drainage is a rare and diverse anomaly, accounting for 1% to 3% of patients with congenital heart disease. Newborns with diagnosis of an obstructed total anomalous pulmonary venous dainage are extremely ill soon after birth and often present with severe cyanosis, pulmonary hypertension and low cardiac output requiring urgent surgical intervention. Transcatheter palliative stenting of the obstructive vertical vein can be an acceptable alternative as a bailout intervention before complete surgical correction is undertaken. This report of two cases highlights the feasibility, safety and effectiveness of the inter ventional palliative procedure and confirms that this technique can be an acceptable and attractive bridge in the algorithm of medical decisions during the evaluation of these critical patients.

Resumen El drenaje venoso pulmonar anómalo total es una enfermedad poco frecuente y de presentación diversa y se observa en el 1% a 3% de las cardiopatías congénitas. Si se asocia a obstrucción, se convierte en una afección grave en el recién nacido, mostrando cianosis intensa, hipertensión arterial pulmonar y bajo gasto cardíaco con indicación de intervención quirúrgica de urgencia. El implante de stent por cateterismo de forma paliativa para aliviar la obstrucción puede ser una alternativa aceptable de tratamiento como intervención de rescate antes de la corrección quirúrgica definitiva. Presentamos dos casos de intervención percutánea paliativa mostrando que esta técnica puede ser eficaz como puente al tratamiento quirúrgico definitivo para ser incorporado en la toma de decisiones de estos pacientes críticos.

Magnetic resonance lymphangiography in group 1 paediatric pulmonary arterial hypertension.

Pulmonary hypertension could have thoracic lymphatic abnormalities caused by right ventricular failure. Since there is no description of such abnormalities, the purpose of this study was to investigate them with magnetic resonance. Prospective review magnetic resonance T2-weighted lymphangiography was performed between January 2017 and October 2019 through quantitative thoracic duct diameter, diameter index and qualitative lymphatic abnormalities types: 1 - little or none abnormalities, 2 - abnormalities in supraclavicular region, 3 - abnormalities extending into the mediastinum and 4 - abnormalities extending into the lung. Five patients with group 1 pulmonary arterial hypertension participated in this study. The mean age was 12.44 ± 4.92 years, three male and two female. The quantitative analysis yielded the following results: mean thoracic duct diameter of 2.92 ± 0.16 mm and thoracic duct index 2.28 ± 1.03 mm/m2. Qualitative lymphangiography abnormalities were type 1 in three patients, type 2 in one, all with low-risk determinants, and type 3 in one with high-risk determinants and right ventricular failure. Magnetic resonance T2-weighted lymphangiography in group 1 paediatric pulmonary arterial hypertension allowed for the identification of the thoracic duct, which was used to perform both quantitative and qualitative analysis of thoracic lymphatic abnormalities, in particular when increased high-risk determinants and right ventricular failure were present. These features represent an extracardiac finding useful to understand systemic venous congestion impact on lymphatic system.